2.01VP Early cardiac involvement in children with Duchenne muscular dystrophy

Duchenne muscular dystrophy is a progressive X-linked disorder in which cardiac involvement has emerged as a major determinant of morbidity with improved survival. Early detection of subclinical myocardial dysfunction is essential for timely initiation of cardio-protective therapy. This hospital-based observational study evaluated cardiac involvement in 37 children with clinically and genetically confirmed Duchenne muscular dystrophy at a tertiary care centre. All participants underwent clinical assessment, electrocardiography, and echocardiography including measurement of left ventricular ejection fraction and global longitudinal strain. Most children were aged 6–10 years (48.6%) or older than 10 years (45.9%). Cardiac involvement was identified in 40.5% of patients, increasing to 64.7% in those above 10 years. Electrocardiographic abnormalities were common, including sinus tachycardia (73%), short PR interval (44.1%), deep Q waves (59.5%), and abnormal R/S ratio in lead V1 (63%). Although left ventricular ejection fraction was preserved in most patients (86.1%), abnormal global longitudinal strain was observed in 93.8%, indicating early myocardial dysfunction not detected by conventional measures. These findings demonstrate that cardiac involvement in Duchenne muscular dystrophy is common, age-related, and frequently subclinical, and that strain imaging may enable earlier detection and guide timely cardio-protective intervention to improve long-term outcomes.